Benign myoclonic epilepsy in infancy: neuropsychological and behavioural outcome.

Benign myoclonic epilepsy in infancy (BMEI) is a rare syndrome of idiopathic generalized epilepsies with onset below 3 years of age. It has been reported that BMEI is associated with a good prognosis, however, recently some studies suggest less favourable neuropsychological outcome. We report a long-term follow-up of seven patients with BMEI. Seizure outcome and neuropsychological, cognitive, and behavioural evolution were discussed for each of them. At the end of follow-up, 86% of children showed neuropsychological and intellectual disorders: two children had mental retardation, three patients achieved a borderline IQ and one normal but low IQ. All but one displayed neuropsychological disabilities including fine motor skill deficits, attention deficits, and language impairment and learning disorders. Our clinical data and the previous reports suggest that the early onset of the seizures may be one of the main factors of the illness giving rise to a less favourable outcome. Additional interacting factors such as delayed start of treatment, and efficacy of the drugs may play an important role, too. We believe that BMEI does not exert, different from some epileptic encephalopathies, a quick destroying effect but may interfere with the growth of developing functions, which results in long-term neuropsychological disabilities.

Non-convulsive status epilepticus associated with tiagabine in a pediatric patient.

We report a 4-year-old patient who developed non-convulsive status epilepticus (NCSE) following tiagabine (TGB) as add-on treatment for refractory partial seizures. NCSE occurred while the patient received TGB 0.83 mg/kg/day. In our case, the TGB reduction led to a significant improvement of electroclinical features. The mechanisms of this abnormal effect are not clear. GABA-ergic hyperfunction and/or multiplicity of interlinked brain GABA systems associated with individual specific sensitivity could play a critical role in the pathogenesis of NCSE. This is the first report of NCSE documented by electroencephalogram (EEG) in a child under 12 years of age on TGB treatment.